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Deformational Plagiocephaly, Brachycephaly, and Scaphocephaly

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Deformational Plagiocephaly, Brachycephaly, and Scaphocephaly ORIGINAL ARTICLE Deformational Plagiocephaly, Brachycephaly, and Scaphocephaly. Part I: Terminology, Diagnosis, and Etiopathogenesis Gary F. Rogers, MD, JD, MBA, MPH oblique cranial length ratio of 106% or greater, and brachycephaly as Abstract: Cranial deformation is the most common cause of ab- a cephalic index (CI = cranial width/length) of 93% or greater. Using normal head shape. Intentional and unintentional alterations of these definitions, the combined prevalence of flattening was as high cranial form are associated with the application of external pressure as 19.7% at age 4 months and declined to 3.3% by 2 years of age. to the growing infant head, and such changes have been recorded However, when flattening was defined as an oblique cranial length throughout man’s history. Recent changes in Western sleeping ratio of 105% or greater and CI of 91% or greater, the prevalence practices, instituted to reduce the incidence of sudden infant death increased to 28% at 4 months and 12.7% at 2 years. There has been syndrome, have led to a dramatic rise in the incidence of cranial even more variation in the methods used to measure and report deformation and renewed interest in this subject. This 2-part review flattening, as discussed below. presents a pragmatic clinical approach to this topic including a critical review of the literature as it applies to each aspect of this DIAGNOSIS AND TERMINOLOGY common diagnosis: historical perspective, terminology, differential diagnosis, etiopathogenesis and predisposing factors, and prevention Deformational Plagiocephaly and treatment. Deformational cranial flattening can take many forms, de- pending on the position of the infant’shead during the first few months Key Words: Deformational plagiocephaly, deformational of life. Most clinicians incorrectly refer to any type of cranial flattening brachycephaly, deformational scaphocephaly, congenital muscular as Bplagiocephaly.[ Plagiocephaly is derived from the Greek plagios torticollis, craniosynostosis, cranial deformation meaning Boblique[ or Bslanted,[ and kephale¯,meaningBhead.[ Thus, the term deformational plagiocephaly is correctly applied to describe (J Craniofac Surg 2011;22: 9Y16) only flattening that is on one side of the head (Fig. 1). Deformational plagiocephaly occurs primarily in infants who consistently favor BACKGROUND turning their head to one side, that is, those with congenital muscular 17 The American Academy of Pediatrics initiated the BBack to torticollis (CMT). The resultant cranial shape has been compared 18 Sleep Campaign[ in 1992 based on a mounting body of evidence with a Bparallelogram[; however, the frontal bossing is never equal that supine positioning of infants during sleep may reduce the to the degree of occipital flattening, and thus, the shape is really more incidence of sudden infant death syndrome.1Y3 This policy has trapezoidal. Asymmetric growth of the head often is accompanied by been widely implemented in North America and resulted in a facial asymmetry, specifically an anterior shift of the ipsilateral fore- 40% reduction in the incidence of sudden infant death syndrome head, ear, and cheek (Fig. 2). Asymmetric opening of the palpebral in the United States.4 One of the unforeseen consequences of the fissures can also be observed as a consequence of the sagittal dis- campaign was an exponential rise in asymmetric (plagiocephaly) placement of the ipsilateral zygoma. As asymmetric occipital flatten- and symmetric (brachycephaly) occipital flattening.5Y8 Similar cra- ing progresses, forward movement of the zygoma and attached lateral nial shape changes (frontal plagiocephaly) had historically been canthus on the affected side effectively shortens the distance between observed in infants who slept prone,9Y14 but the prevalence and the medial and lateral canthal tendons. As a result, tension is reduced degree of flattening were considerably less. Recent studies estimate on the tarsal plates, and the eye appears more open on the side of the the prevalence of deformational posterior cranial flattening to be as flattening (Fig. 3). The vertical palpebral asymmetry can be easily high as 18% to 19.7% in healthy infants15; these calculations vary, confused with contralateral eyelid ptosis. Deformational plagioce- depending on how this entity is defined.16 For example, Hutchison phaly must be distinguished from 2 types of craniosynostosis that also and coworkers15 followed up 200 normal infants from birth to can cause an asymmetric head shape: unilateral coronal synostosis 2 years of age. They defined deformational plagiocephaly (DP) as an (UCS) and lambdoidal synostosis. Both conditions are rare compared with deformational flattening. Unilateral coronal synostosis, or pre- mature closure of 1 coronal suture, causes anterior plagiocephaly. From the Department of Plastic and Oral Surgery, Children’s Hospital Boston, Features of this entity include flattening of the forehead and superior and Harvard Medical School, Boston, Massachusetts. orbital rim such that the anterior globe protrudes beyond these struc- Received April 27, 2010. tures, nasal root and midfacial angulation, and anterior displacement of Accepted for publication June 4, 2010. the ear ipsilateral (Figs. 4A, B). These features are not seen in DP. Address correspondence and reprint requests to Gary F. Rogers, MD, JD, Asymmetry of the palpebral fissures in UCS can look similar to that MBA, MPH, 300 Longwood Ave, Boston, MA 02115; E-mail: Y [email protected] seen in severe DP. However, the more-open appearing eye in UCS is The author has an ownership interest in PlagioPrevention, LLC. on the side of the flat forehead, whereas in DP it is on the side of Copyright * 2011 by Mutaz B. Habal, MD increased forehead bossing. Lambdoidal synostosis, or synostotic ISSN: 1049-2275 posterior plagiocephaly, can be difficult to differentiate from DP.This DOI: 10.1097/SCS.0b013e3181f6c313 condition is associated with asymmetric cranial height (shorter on the The Journal of Craniofacial Surgery & Volume 22, Number 1, January 2011 9 Copyright © 2011 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited. Rogers The Journal of Craniofacial Surgery & Volume 22, Number 1, January 2011 FIGURE 1. Right DP, vertex view. Note that shape is more FIGURE 3. Right DP, frontal view. Forehead and cheek trapezoidal and not a true parallelogram as often stated. more prominent on the right than the left; right ear is anterior flattened side, the opposite of DP) that gives a Bwind-swept[ appear- relative to the left in the sagittal plane; right eye appears ance to the head, and there is usually mastoid bossing on the affected more open than left; chin point rotated to left. Nose is straight. side (Figs. 5 and 6). infants who have extreme head rotation to one side or in premature Deformational Brachycephaly infants who are positioned side-to-side in the intensive care units Brachycephaly (Greek brachy, meaning Bshort[) denotes (Fig. 10). Flattening develops on the side(s) of the head, and com- symmetrical occipital flattening and compensatory parietal widen- pensatory expansion occurs in the anterior and posterior cranium. ing. Infants with deformational brachycephaly (DB) have little or These infants tend to develop a long, slender head, colloquially no rounding on the back of the head and appear to have a dispro- referred to by some as a Btoaster head.[ There is often relatively portionately wide or Bbig[ head viewed from the front (Fig. 7A). pronounced facial asymmetry. This presentation can be confused The posterior vertex may appear taller than the front (turricephaly), with scaphocephaly caused by premature fusion of the sagittal giving a sloped appearance to the head in profile (Fig. 7B). The ratio suture. Unlike DS, sagittal synostosis typically results in frontal of cranial width to length, termed the cranial index or CI, is gener- bossing, bilateral occipital/parietal narrowing posterior to the anterior ally higher than normal (Fig. 7C)Vthis figure is historically 0.75 to fontanelle, and decreased vertical height of the posterior cranium 0.80 in North America, although some observers suggest that (Figs. 11A, B). Facial asymmetry is rare in sagittal synostosis. Addi- the normal CI has risen to 0.8 to 0.85 in response to back sleep- tionally, most infants with this type of craniosynostosis have a head ing.19 Most children with DB also have some element of asymme- circumference in excess of the 90th percentile. try, or plagiocephaly. The combination effect, which I refer to as Basymmetric brachycephaly,[ is the most common type of de- Radiographic Imaging formational shape (Fig. 8). Brachycephaly can also be seen in in- If the diagnosis is unclear, the child should be referred to fants with craniosynostosis when both coronal sutures are fused. a specialist before ordering radiographic studies. The accuracy of Synostotic brachycephaly is relatively rare and has features not plain radiography to diagnose suture fusion is questionable. Com- seen in DB: severe forehead retrusion such that the superior orbital puted tomography is costly, often requires sedation, and involves rim is behind the anterior surface of the globe (eyes appear very prominent) and anterior turricephaly (Btall[ head) (Figs. 9A, B). Deformational Scaphocephaly Deformational scaphocephaly (DS) (Bboatlike head[)isan uncommon variant of plagiocephaly. It is more commonly seen in FIGURE 4. Right UCS. A, Frontal view. Similarities to DP include right eye appears more open than left eye, anterior displacement
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